Lack of methylation changes in GJB2 and RB1 non-coding regions of cochlear implant patients with sensorineural hearing loss

Objective@#Recent advances in epigenetic studies continue to reveal novel mechanisms of gene regulation and control, however little is known on the role of epigenetics in sensorineural hearing loss (SNHL) in humans. We aimed to investigate the methylation patterns of two regions, one in RB1 and another in GJB2 in Filipino patients with SNHL compared to hearing control individuals. @*Methods@#We investigated an RB1 promoter region that was previously identified as differentially methylated in children with SNHL and lead exposure. Additionally, we investigated a sequence in an enhancer-like region within GJB2 that contains four CpGs in close proximity. Bisulfite conversion was performed on salivary DNA samples from 15 children with SNHL and 45 unrelated ethnically-matched individuals. We then performed methylation-specific real-time PCR analysis (qMSP) using TaqMan® probes to determine percentage methylation of the two regions. @*Results@#Using qMSP, both our cases and controls had zero methylation at the targeted GJB2 and RB1 regions. @*Conclusion@#Our study showed no changes in methylation at the selected CpG regions in RB1 and GJB2 in the two comparison groups with or without SNHL. This may be due to a lack of environmental exposures to these target regions. Other epigenetic marks may be present around these regions as well as those of other HL-associated genes.

Maternal diabetes: A potential risk factor of congenital hearing loss

@#Objective. The purpose of this study is to identify the incidence rate of 'refer' result in neonates born to diabetic mothers and to determine the association of maternal diabetes and the initial 'refer' result. Methods. This was a retrospective cross-sectional study which included neonates who had hearing screening test using transient-evoked otoacoustic emissions test (TEOAE) on both ears at the Philippine General Hospital Ear unit during three weeks. We obtained the demographic characteristics, presence/absence of maternal diabetes, and OAE results. Results. Among the 150 neonates, ten were born to diabetic mothers, with an age range of 2-8 days old. Forty percent of neonates of diabetic mothers had an initial 'refer' result compared with 7.9% of nondiabetic mothers' neonates. After logistic regression analysis, there is a significant association between maternal diabetes and initial 'refer' result in OAE with a p-value <0.05. If the mother is diagnosed with diabetes (gestational/pre-gestational), the odds of having an initial 'refer' result in the hearing screening is 2x higher. The odds can range from 2-43 times. Conclusion. The incidence rate of an initial 'refer' result in neonates of diabetic mothers is 40%. There is a significant association between maternal diabetes and the initial 'refer' result in the OAE test.

Evaluation of the newborn hearing screening program in The Medical City based on Joint Commission on Infant Hearing (JCIH) 2007 position statement quality indicators

Objective@#The objective of this study is to evaluate the newborn hearing screening program in The Medical City based on the Joint Committee on Infant Hearing (JCIH) 2007 Position Statement Quality Indicators. @*Methods@#Study Design: Cross - Sectional Survey. Setting: Tertiary Private Hospital. Participants: All newborns who underwent newborn hearing screening in The Medical City for the year 2015. @*Results@#Of 2,010 patients delivered in the hospital in year 2015, 1,986 (98.8%) were screened. Among the 59 babies with initial “refer” results, 15 (25.42%) “referred” a second time while 24 (40.68%) “passed” the rescreening. Twenty (33.89%) did not undergo rescreening (10 were classified as dropouts, while another 10 did not undergo rescreening for various reasons. Of those who “referred” during rescreening, only 9 (60%) had further evaluation done with ABR/ASSR. Among these, 4 (26.66%) had hearing loss and proceeded with the appropriate monitoring and management while 5 (33.33%) had normal hearing. @*Conclusion@#The current newborn hearing screening program in the Medical City was able to reach JCIH 2007 quality indicators for screening but not for confirmation of hearing Loss. All patients with hearing loss were managed with early rehabilitation.

The cost-effectiveness and budget impact of a community-based universal newborn hearing screening program in the Philippines

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> In 2000, the World Health Organization recommended implementation of universal hearing screening. The Philippines enacted this policy into law in 2009 as it was found to be cost-effective for the Philippines. The model at the time used a hospital-based approached to screening. This paper examines the cost-effectiveness and budget impact of implementing a community-based universal hearing screening program.<br /><strong>METHODS:</strong> A model was developed following a community-based hearing screening program. Parameters were obtained through literature review, secondary data analysis, and consultation with experts. Cost-effectiveness was assessed for a single birth cohort from a public payer and societal perspective.<br /><strong>RESULTS:</strong> A community-based universal hearing screening program was found to be cost-saving. One-way sensitivity analysis showed that results were sensitive only to treatment rate and follow-up rate. The program is also a high budget impact program.<br /><strong>CONCLUSION:</strong> A community-based hearing screening program is cost-saving for the Philippines. Ensuring treatment and good follow-up in testing will ensure cost-effectiveness.</p>

Initial otoacoustic emission hearing screening results in newborns with patent ear canals, vernix caseosa and collapsed ear canals

@#<p style="text-align: justify;"><strong>OBJECTIVES: </strong>This study determined the initial otoacoustic (OAE) hearing screening results of newborns with collapsed ear canals and vernix caseosa in the ear canal and compared these to ears that were patent.<br /><strong>METHODS:</strong> Two hundred term newborns (400 ears) with normal APGAR scores, birth weight, maternal and gestational history, who were born between August 2013 to October 2013 and who had OAE hearing screening test done by trained midwives were included in this study. All of them underwent otoscopy after the OAE hearing screening test was done to determine patency of the ear canal and presence of vernix caseosa. The examining physician was blinded to the OAE results. Comparison between the OAE results and the otoscopic findings were done.<br /><strong>RESULTS:</strong> Four hundred ears were included in the study. Two hundred and fifty one ears (62.8%) had vernix caseosa and 42 ears (10.5%) had collapsed ear canal. The overall initial OAE hearing screening test pass rate of the newborns tested was 69.5%. The initial OAE hearing screening test pass rate of newborns those with ear canal vernix caseosa or collapsed ear canal, were 72.1% and 47.6%, respectively. Patent ears were found in 107 (26.7%) with a pass rate of 71.9%.<br /><strong>CONCLUSION:</strong>The pass rates of ears with vernix caseosa and collapsed ear canal were 72.1% and 47.6%, respectively. There was no significant difference between the OAE hearing screening test pass rates of ears with patent canal and ears that were collapsed and/ or had vernix caseosa. However, there was a statistically significant difference in pass rates between patent ear canals and collapsed ear canal</p>

Maternal emotional reactions towards results of newborn hearing screening: A cross sectional survey

@#<p style="text-align: justify;"><strong>OBJECTIVES:</strong> This study aims to determine the maternal reactions and emotions towards an initial "refer" result in the newborn hearing screening test and to determine the proportion of mothers who fully comprehended the test results of the newborn hearing screening test.<br /><strong>METHODS:</strong> In this study, mothers of newborns with a "refer" result in the Universal Newborn Hearing Screening (UNHS) are given questionnaires which cover their understanding of the hearing screening test, the emotions they felt and attitude towards the results.<br /><strong>RESULTS:</strong> Our findings showed that the UNHS program protocol was properly understood by almost all the respondent mothers. Of the emotions listed from the questionnaire, being "worried" was the most persistently felt emotion.<br /><strong>CONCLUSION:</strong> It is important that the results of the newborn hearing screening test be properly understood by the mothers. Mothers who experienced negative emotions brought about by the hearing screening test still believed that the newborn hearing screening test was important, were glad that their child underwent the newborn hearing screening test and will request it for their future offspring.</p>

Comparative study of the auditory steady-state response (ASSR) and click auditory brainstem-evoked response (ABR) thresholds among filipino infants and young children

Objective: To compare the results of auditory steady-state response (ASSR) and click auditory brainstem response (click ABR) among infants and young children tested at the Ear Unit of a Tertiary General Hospital. Methods: Design: Cross-sectional Study Setting: Tertiary General Hospital Population: Within-subject comparisons of click auditory brainstem response (click ABR) thresholds and auditory steady-state response (ASSR) thresholds among 55 infants and young children, 2 months to 35 months of age referred to the Ear Unit for electrophysiologic hearing assessment. Results: Click ABR showed strong positive correlation to all frequencies and averages of ASSR. Highest correlation was noted with the average of 1-4 kHz ASSR results with Pearson r = 0.89 (Spearman r=0.80), the average of 2-4 kHz had strong positive correlation r = 0.88 (0.79). Correlation was consistently strong through all ASSR frequencies (0.5 kHz at r=0.86 (0.74), 1 kHz at r=0.88 (0.78), 2 kHz at r=0. 87 (0.79), 4 kHz at r=0.85 (0.76)). Average differences of click ABR and ASSR thresholds were 8.2±12.9dB at 0.5 kHz, 8.6±12.6dB at 1 kHz, 5.3±11.8dB at 2 kHz and 7.8±13.4dB at 4 kHz. Among patients with no demonstrable waveforms by click ABR with maximal click stimulus, a large percentage presented with ASSR thresholds. Of these, 80.5% (33 of 41) had measurable results at 0.5 kHz with an average of 107.3±11.1dB, 85.4% (35 of 41) at 1 kHz with an average of 110.5±11.8dB, 73.2% (30 of 41) at 2 kHz with an average of 111.2±11.1dB and 63.4% (26 of 41) at 4 kHz with average of 112.2±8.21dB. Auditory steady-state response results were comparable to auditory brainstem response results in normal to severe hearing loss and provided additional information necessary for complete audiologic assessment especially among patients with severe to profound hearing loss wherein click ABR showed no responses. Up to 85.4% of patients that would have been noted to have no waveforms by click ABR still demonstrated measurable thresholds by ASSR. Conclusion: Our study suggests that ASSR may be the best available tool for assessing children with severe to profound hearing loss, and is a comparably effective tool in overall hearing assessment for patients requiring electrophysiological testing. The advantages of ASSR over click ABR include: 1) detection of frequency-specific thresholds and; 2) the detection of hearing loss thresholds beyond the limits of click ABR.

Fractured tracheostomy tube ingestion in a pediatric patient

Objective:To report a case of fractured tracheotomy tube ingestion in a pediatric patient, discussing the clinical presentation, complications and management of tracheobronchial tree versus upper digestive tract foreign bodies. Methods: Design: Case Report Setting: Tertiary Private Hospital Patient: One Result: A 4-year-old male child with unusual hypersensitivity to routine tracheotomy suctioning was discovered to have a fractured tracheotomy tube. Emergency radiographs localized the cannula in the abdomen and bronchoscopy was deferred. The foreign body was eventually passed out after four days. Conclusion: Due diligence in diagnostics prior to bronchoscopy led to the avoidance of an unnecessary and sometimes complicated procedure. In developing countries with poor access to health care, the importance of regular tracheotomy follow-ups and periodic replacement cannot be overemphasized. A search of the English literature using Pubmed and Ovid search engines with keywords tracheostomy, foreign bodies and pediatrics confirms that this is the first reported accidental ingestion of a fractured tracheotomy tube in a pediatric patient.